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Epidermal nevus syndrome
Susheera Chatproedprai1, Siriwan Wananukul, Thanaboon Prasarnnaem
1Department of Pediatrics, Faculty of Medicine, Chulalongkorn University and Hospital, Bangkok, Thailand. susheerac@hotmail.com
International Journal of Dermatology
|July 27, 2007
Summary
Epidermal nevus syndrome, a rare congenital condition, involves skin abnormalities and can affect the brain and eyes. This case highlights an unusual presentation of inflammatory linear verrucous epidermal nevus on the head.
Area of Science:
- Dermatology
- Neurology
- Genetics
Background:
- Epidermal nevus syndrome (ENS) is a spectrum of congenital neurocutaneous disorders.
- It is characterized by epidermal nevi and associated extracutaneous anomalies.
- Classification of nevi is based on their predominant histological component.
Observation:
- A case report of a child with ENS is presented.
- The child exhibited an inflammatory linear verrucous epidermal nevus (ILVNE) located on the head.
- This location is considered uncommon for ILVNE.
Findings:
- The patient presented with an ILVNE on the scalp.
- Associated findings included structural disorders of the central nervous system (CNS).
- Ocular abnormalities were also noted in the patient.
Implications:
- This case expands the understanding of ENS presentations.
- It emphasizes the importance of considering CNS and ocular evaluations in patients with atypical epidermal nevi.
- Highlights the diagnostic challenges and management considerations for rare congenital conditions.
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